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NF1-Associated Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1709893
Concept ID:
C5236143
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor that is associated with NF1 gene inactivation and a history of neurofibromatosis type 1. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNF1-Associated Malignant Peripheral Nerve Sheath Tumor

Recent clinical studies

Etiology

Mitchell DK, Burgess B, White EE, Smith AE, Sierra Potchanant EA, Mang H, Hickey BE, Lu Q, Qian S, Bessler W, Li X, Jiang L, Brewster K, Temm C, Horvai A, Albright EA, Fishel ML, Pratilas CA, Angus SP, Clapp DW, Rhodes SD
Clin Cancer Res 2024 Mar 1;30(5):1038-1053. doi: 10.1158/1078-0432.CCR-23-2548. PMID: 38127282Free PMC Article
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
Rodriguez FJ, Graham MK, Brosnan-Cashman JA, Barber JR, Davis C, Vizcaino MA, Palsgrove DN, Giannini C, Pekmezci M, Dahiya S, Gokden M, Noë M, Wood LD, Pratilas CA, Morris CD, Belzberg A, Blakeley J, Heaphy CM
Acta Neuropathol Commun 2019 Aug 28;7(1):139. doi: 10.1186/s40478-019-0792-5. PMID: 31462295Free PMC Article
Fischer-Huchzermeyer S, Dombrowski A, Wilke G, Stahn V, Streubel A, Mautner VF, Harder A
PLoS One 2017;12(11):e0187700. Epub 2017 Nov 13 doi: 10.1371/journal.pone.0187700. PMID: 29131833Free PMC Article
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406

Diagnosis

Mitchell DK, Burgess B, White EE, Smith AE, Sierra Potchanant EA, Mang H, Hickey BE, Lu Q, Qian S, Bessler W, Li X, Jiang L, Brewster K, Temm C, Horvai A, Albright EA, Fishel ML, Pratilas CA, Angus SP, Clapp DW, Rhodes SD
Clin Cancer Res 2024 Mar 1;30(5):1038-1053. doi: 10.1158/1078-0432.CCR-23-2548. PMID: 38127282Free PMC Article
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Tritto V, Ferrari L, Esposito S, Zuccotti P, Bianchessi D, Natacci F, Saletti V, Eoli M, Riva P
Genes (Basel) 2019 Nov 5;10(11) doi: 10.3390/genes10110892. PMID: 31694342Free PMC Article
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Therapy

Borcherding DC, Amin NV, He K, Zhang X, Lyu Y, Dehner C, Bhatia H, Gothra A, Daud L, Ruminski P, Pratilas CA, Pollard K, Sundby T, Widemann BC, Hirbe AC
Clin Cancer Res 2023 Apr 14;29(8):1592-1604. doi: 10.1158/1078-0432.CCR-22-3722. PMID: 36799629Free PMC Article
Galvin R, Watson AL, Largaespada DA, Ratner N, Osum S, Moertel CL
Curr Oncol Rep 2021 Mar 15;23(4):45. doi: 10.1007/s11912-021-01032-y. PMID: 33721151Free PMC Article
Schulte A, Ewald F, Spyra M, Smit DJ, Jiang W, Salamon J, Jücker M, Mautner VF
Int J Mol Sci 2020 Feb 24;21(4) doi: 10.3390/ijms21041548. PMID: 32102484Free PMC Article
Cooper JM, Patel AJ, Chen Z, Liao CP, Chen K, Mo J, Wang Y, Le LQ
Clin Cancer Res 2019 Jun 1;25(11):3404-3416. Epub 2019 Feb 22 doi: 10.1158/1078-0432.CCR-18-2437. PMID: 30796033Free PMC Article
Varin J, Poulain L, Hivelin M, Nusbaum P, Hubas A, Laurendeau I, Lantieri L, Wolkenstein P, Vidaud M, Pasmant E, Chapuis N, Parfait B
Oncotarget 2016 Jun 14;7(24):35753-35767. doi: 10.18632/oncotarget.7099. PMID: 26840085Free PMC Article

Prognosis

Larsson AT, Bhatia H, Calizo A, Pollard K, Zhang X, Conniff E, Tibbitts JF, Rono E, Cummins K, Osum SH, Williams KB, Crampton AL, Jubenville T, Schefer D, Yang K, Lyu Y, Pino JC, Bade J, Gross JM, Lisok A, Dehner CA, Chrisinger JSA, He K, Gosline SJC, Pratilas CA, Largaespada DA, Wood DK, Hirbe AC
Neuro Oncol 2023 Nov 2;25(11):2044-2057. doi: 10.1093/neuonc/noad097. PMID: 37246765Free PMC Article
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
Sohier P, Luscan A, Lloyd A, Ashelford K, Laurendeau I, Briand-Suleau A, Vidaud D, Ortonne N, Pasmant E, Upadhyaya M
Genes Chromosomes Cancer 2017 May;56(5):421-426. Epub 2017 Mar 7 doi: 10.1002/gcc.22446. PMID: 28124441
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Clinical prediction guides

Larsson AT, Bhatia H, Calizo A, Pollard K, Zhang X, Conniff E, Tibbitts JF, Rono E, Cummins K, Osum SH, Williams KB, Crampton AL, Jubenville T, Schefer D, Yang K, Lyu Y, Pino JC, Bade J, Gross JM, Lisok A, Dehner CA, Chrisinger JSA, He K, Gosline SJC, Pratilas CA, Largaespada DA, Wood DK, Hirbe AC
Neuro Oncol 2023 Nov 2;25(11):2044-2057. doi: 10.1093/neuonc/noad097. PMID: 37246765Free PMC Article
Rodriguez FJ, Graham MK, Brosnan-Cashman JA, Barber JR, Davis C, Vizcaino MA, Palsgrove DN, Giannini C, Pekmezci M, Dahiya S, Gokden M, Noë M, Wood LD, Pratilas CA, Morris CD, Belzberg A, Blakeley J, Heaphy CM
Acta Neuropathol Commun 2019 Aug 28;7(1):139. doi: 10.1186/s40478-019-0792-5. PMID: 31462295Free PMC Article
Fischer-Huchzermeyer S, Dombrowski A, Wilke G, Stahn V, Streubel A, Mautner VF, Harder A
PLoS One 2017;12(11):e0187700. Epub 2017 Nov 13 doi: 10.1371/journal.pone.0187700. PMID: 29131833Free PMC Article
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

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